Non-immune fetal hydrops is diagnosed when there is fluid accumulation in more than one extravascular space. A long list of etiologies has been found in Etiology of non-immune · Metabolic disorders as a · Diagnosis of a metabolic. Jump to Non-immune - The non-immune form of hydrops fetalis has many causes in which fetal and placental hydrops develops in association with. Nonimmune hydrops fetalis is a rare disorder that is often diagnosed prenatally. It is characterized by an abnormal accumulation of fluid in fetal tissues and body.
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The classic diagnosis is usually done by measuring the level of the specific enzyme.
Different nonimmune fetal hydrops storage nonimmune fetal hydrops are associated with different enzyme deficiency. Examples include beta-glucuronidase deficiency in Mucopolysaccharidosis type VII, beta-galactosidase deficiency in GM1 gangliosidosis.
Other methods of diagnosis include histological examination of different organs such as liver, spleen, lungs, bone marrow, or placenta 15 Mutation identification is not available for all diseases, and in some diseases the parents might not be carrying the common mutation for that particular disorder, which could limit the possibility of prenatal diagnosis Another difficulty that is faced in some diseases is that there are cases of pseudodeficiency in the enzyme which might be common in a particular disease example include metachromatic leucodystrophy.
In these cases it is nonimmune fetal hydrops to know the enzyme level in the parents and the index case, or the mutation in the family before prenatal diagnosis can be offered Because of these issues, In-utero diagnosis of a metabolic disorder in the absence of an index case is difficult and only available in selected laboratories around the world.
Few numbers of cases of Mucopolysaccharidosis type VII have been reported with prenatal diagnosis, by either amniocentesis or chorionic villus sampling, most of which there was a previously affected child in the family 1019 It is therefore of ultimate nonimmune fetal hydrops to try to identify the cause of non-immune hydrops in the index case, if not antenatal, then postnatal.
This will be helpful when dealing with these families preconception, in order to provide them with appropriate counseling and options for next pregnancy.
Non-immune fetal hydrops: Are we doing the appropriate tests each time?
Screening for metabolic abnormalities should be considered in any work-up for non-immune hydrops fetalis, but with repeat hydrops it should be mandatory. Large nuchal translucency and metabolic disorders Nuchal translucency, which is a measurement of the fluid accumulation in nonimmune fetal hydrops back of the fetal neck has been found to be increased not only in fetuses with chromosomal abnormalities, but also in a variety of fetal abnormalities including structural fetal defects and genetic diseases Since that time, many reports came out to indicate that metabolic disorders can be suspected if increased nuchal translucency is diagnosed in the first trimester.
Many metabolic diseases have been found to have this association including Zellweger syndrome 22 — 24mucopolysaccharidosis type VII 25 Obviously, not all lysosomal storage diseases present with enlarged nuchal translucency, a recent study by De Biasio et al demonstrated that nuchal translucency nonimmune fetal hydrops be normal in these fetuses Conclusions Metabolic disorders, although uncommon, must be always thought of when investigating cases of non-immune hydrops, and enlarged nuchal translucency.
Depending on the population managed, these diseases are much more commonly present than what we might think. Routine screening for metabolic diseases especially lysosomal storage diseases should be considered in at risk population, and definitely in cases of recurrent hydrops in the same family.
Every effort should nonimmune fetal hydrops made to identify the etiology in an index case in the family as this might be the best opportunity for improving future care.
Non-immune hydrops fetalis-diagnosis and management.
The obstetric management of non-immune Hydrops. Medicine to cause early labor and delivery of the baby Early nonimmune fetal hydrops delivery if condition gets worse Giving blood to the baby while still in the womb intrauterine fetal blood transfusion Treatment for a newborn may include: For immune hydrops, direct transfusion of red blood cells nonimmune fetal hydrops match the infant's blood type.
An exchange transfusion to rid the baby's body of the substances that are destroying the red blood cells is also done. Removing extra nonimmune fetal hydrops from around the lungs and abdominal organs with a needle.
Hydrops fetalis - Wikipedia
Medicines to control heart failure and nonimmune fetal hydrops the kidneys remove extra fluids. In response to increased central venous pressure In response to increased arterial pressure In response to obstructed venous drainage Results from primary or secondary heart failure.
Results from obstruction of venous return. Increased capillary pressure causes excessive filtration of fluid into the interstitial tissues.
Fetal echocardiogram Consider fetal heart rate monitoring for 12 nonimmune fetal hydrops 24 hours if fetal arrhythmia is suspected. Amniocentesis for fetal karyotype and PCR polymerase chain reaction for infections OR fetal percutaneous blood sampling for same and in addition fetal liver function; and metabolic testing if indicated.